Hearing impairment has long been recognized as a common feature in osteogenesis imperfecta. The figures in some publications could be taken to imply that, with increasing age, the proportion of osteogenesis imperfecta patients with hearing impairment approaches 100 per cent.

The incidence of hearing loss in a large survey of 1394 patients with osteogenesis imperfecta was examined. It was found that the most common age of onset was in the second, third and fourth decades of life. At the age of 50 approximately 50 per cent of the patients had symptoms of hearing impairment; over the next 20 years there was little further increase.

Differences were shown between patients with different clinical types of osteogenesis imperfecta as delineated in the Sillence classification; hearing loss was significantly less common in the type IV disease than in the type I disorder. Among the 29 families with osteogenesis imperfecta type IA there were distinct differences in the likelihood of hearing loss. These findings provide insights which will be valuable in giving patients advice on the likelihood of developing hearing loss in the future.

People with learning disabilities are at increased risk of impaired hearing. The aims of this study were to assess the prevalence of hearing impairment and ear disease in people attending the specialist Otolaryngology/Hearing Therapy clinic at the Phoenix NHS Trust, Bristol. The present and future process of such a service was explored. Data were obtained from the referral form and notes made by the consultant otolaryngologist. In 20 months, there were 226 consultations, 188 of which were new referrals. The majority of patients had verbal communication to some extent. Suspected infection/inflammation and unobtainable/abnormal tympanograms, each accounted for 43 per cent of reasons for referral. Twenty per cent of patients were normal otologically. Eighteen per cent were provided with hearing aids and nine per cent required surgery. Ten patients underwent brainstem evoked response testing, half of whom had aidable hearing. Our results are comparable to published data of similar units. It is recommended that combined otolaryngology/specialist hearing therapy services are continued and further developed.

It has been suggested that there is considerable unmet need in respect of hearing loss amongst the elderly population, but no routine screening test is currently used in general practice to identify these patients. The aim of this study was to determine whether routine questionnaire screening of the over-65s is a feasible way to identify elderly patients with hearing loss in primary care and whether patients so identified would benefit from hearing aid fitting. A cohort of patients consisting of a sample of 234 individuals aged between 65 and 74, attending a doctor’s surgery over a specified period, received a scored questionnaire to complete based on the Hearing Handicap Inventory for the Elderly Screening test. Hearing aid owners and those with a hearing handicap were identified, and non-aid wearers with handicap offered examination and referral. Those patients who were fitted with aids were assessed after six months for aid usage and persisting handicap. Twenty-five per cent of the patient sample reported a previously undiagnosed hearing handicap. Six months after aid fitting, a reduction in hearing handicap was reported in 79 per cent of these cases and overall aid usage in the population sample had increased from nine per cent to 20 per cent. Routine questionnaire screening in general practice may be worthwhile since it is easy to carry out and the resulting intervention significantly reduces reported hearing handicap.